Read by QxMD icon Read

Childhood hearing loss

Agnieszka Jacoszek, Agnieszka Pollak, Rafał Płoski, Monika Ołdak
Hearing plays a crucial role in human development. Receiving and processing sounds are essential for the advancement of the speech ability during the early childhood and for a proper functioning in the society. Hearing loss is one of the most frequent disabilities that affect human senses. It can be caused by genetic or environmental factors or both of them. Calcium- and integrin-binding protein 2 (CIB2) is one of the recently identified genes, involved in HI pathogenesis. CIB2 is widely expressed in various human and animal tissues, mainly in skeletal muscle, nervous tissue, inner ear, and retina...
October 22, 2016: European Archives of Oto-rhino-laryngology
Sigrid Bosteels, Michel Vandenbroeck, Geert Van Hove
New-born screening programs for congenital disorders and chronic disease are expanding worldwide and children "at risk" are identified by nationwide tracking systems at the earliest possible stage. These practices are never neutral and raise important social and ethical questions. An emergent concern is that a reflexive professionalism should interrogate the ever earlier interference in children's lives. The Flemish community of Belgium was among the first to generalize the screening for hearing loss in young children and is an interesting case to study the public justification of early interventions for families with deaf children...
October 19, 2016: Journal of Bioethical Inquiry
Ana'am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 h) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies...
October 13, 2016: Hearing Research
Janni Niclasen, Carsten Obel, Christian Guldager, Simone Pleinert, Jesper Dammeyer
OBJECTIVE: Negative associations between hearing loss (HL) and behavioural and academic difficulties have been reported. However, most studies are based on small clinical samples. The aim of the present study was to investigate such associations using data from a large-scale non-clinical Danish birth cohort controlling for a large number of relevant confounding factors. METHODS: The study applied data from the Aarhus Birth Cohort's 10-12-year-old follow-up (N = 7599)...
November 2016: International Journal of Pediatric Otorhinolaryngology
Vanessa Duah, Zhen Huang, Stephanie Val, Christie DeMason, Marain Poley, Diego Preciado
BACKGROUND: Chronic otitis media (COM) is one of the most common childhood diseases. Its pathophysiology is complex and multifactorial. The role of specific mucin glycoprotein subtypes in OM is only recently being elucidated. OBJECTIVE: To determine the relationship between middle ear fluid mucins and clinical variables of patients needing tympanostomy tubes (TT). METHODS: Middle ear effusions (MEE) from children receiving TT were collected over a 2-year period...
November 2016: International Journal of Pediatric Otorhinolaryngology
Marie Hully, Juliette Ropars, Laurence Hubert, Nathalie Boddaert, Marlene Rio, Mathieu Bernardelli, Isabelle Desguerre, Valerie Cormier-Daire, Arnold Munnich, Pascale de Lonlay, Louise Reilly, Claude Besmond, Nadia Bahi-Buisson
Mutations in ATP1A3 are involved in a large spectrum of neurological disorders, including rapid onset dystonia parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS), with recent descriptions of overlapping phenotypes. In AHC, a few familial cases of autosomal dominant inheritance have been reported, along with cases of de novo sporadic mutations. In contrast, autosomal dominant inheritance has frequently been associated with RDP and CAPOS...
October 10, 2016: Neurogenetics
Soren Gantt, Francois Dionne, Fred K Kozak, Oran Goshen, David M Goldfarb, Albert H Park, Suresh B Boppana, Karen Fowler
Importance: Congenital cytomegalovirus (cCMV) infection is a major cause of childhood deafness. Most cCMV infections are not diagnosed without newborn screening, resulting in missed opportunities for directed care. Objective: To estimate the cost-effectiveness of universal and targeted newborn cCMV screening programs compared with no cCMV screening. Design, Setting, and Participants: Models were constructed using rates and outcomes from prospective cohort studies of newborn cCMV screening in US postpartum care and early hearing programs...
October 10, 2016: JAMA Pediatrics
Takehiko Ueyama, Yuzuru Ninoyu, Shin-Ya Nishio, Takushi Miyoshi, Hiroko Torii, Koji Nishimura, Kazuma Sugahara, Hideaki Sakata, Dean Thumkeo, Hirofumi Sakaguchi, Naoki Watanabe, Shin-Ichi Usami, Naoaki Saito, Shin-Ichiro Kitajiri
DIAPH1 encodes human DIA1, a formin protein that elongates unbranched actin. The c.3634+1G>T DIAPH1 mutation causes autosomal dominant nonsyndromic sensorineural hearing loss, DFNA1, characterized by progressive deafness starting in childhood. The mutation occurs near the C-terminus of the diaphanous autoregulatory domain (DAD) of DIA1, which interacts with its N-terminal diaphanous inhibitory domain (DID), and may engender constitutive activation of DIA1. However, the underlying pathogenesis that causes DFNA1 is unclear...
October 5, 2016: EMBO Molecular Medicine
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
Junpei Koge, Shintaro Hayashi, Hiroo Yamaguchi, Takahisa Tateishi, Hiroyuki Murai, Jun-Ichi Kira
A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
Annette Weiss, Grit Sommer, Rahel Kasteler, Katrin Scheinemann, Michael Grotzer, Martin Kompis, Claudia E Kuehni
BACKGROUND: Auditory complications are an adverse event of childhood cancer treatment, especially common in children treated with platinum chemotherapy or cranial radiation. Variation between diagnostic childhood cancer groups has rarely been studied, and we do not know if the burden of auditory complications has changed over the last decades. PROCEDURE: Within the Swiss Childhood Cancer Survivor Study, we sent a questionnaire to all survivors who were diagnosed at age 16 years or less between 1976 and 2005...
September 21, 2016: Pediatric Blood & Cancer
Vânia Henriques, Rafaela Teles, Ana Sousa, Roberto Estevão, Jorge Rodrigues, Alexandra Gomes, Francisco Silva, Ângelo Fernandes, Fausto Fernandes
Congenital middle ear malformations are rare. Most part of them are usually associated with other malformations, such as aural atresia, microtia, and dysmorphic craniofacial features. A clinical case of a 24-year-old male with a right-sided conductive hearing loss since his childhood, without craniofacial malformation, is presented. He was proposed for exploratory tympanotomy under the suspicious diagnosis of otosclerosis. The surgery revealed an abnormal location of stapes' superstructure, which was attached to the promontory and had an isolated and mobile osseous footplate in the oval window...
2016: Case Reports in Otolaryngology
Eric M Sugihara, Seilesh C Babu, Dennis J Kitsko, Michael S Haupert, Prasad J Thottam
OBJECTIVE: To determine the pediatric incidence and association of superior semicircular canal dehiscence (SSCD) with inner ear (IE) anomalies. STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary referral centers. PATIENTS: Children less than 18 years who received a 0.5 mm or less collimated computed tomography study including the temporal bones between 2010 and 2013 for reasons including, but not limited to, hearing loss, trauma, and infection...
October 2016: Otology & Neurotology
Hikaru Kanemasa, Ryoko Fukai, Yasunari Sakai, Michiko Torio, Noriko Miyake, Sooyoung Lee, Hiroaki Ono, Satoshi Akamine, Kei Nishiyama, Masafumi Sanefuji, Yoshito Ishizaki, Hiroyuki Torisu, Hirotomo Saitsu, Naomichi Matsumoto, Toshiro Hara
BACKGROUND: Alternating hemiplegia of childhood (AHC) is a rare neurological disorder that manifests recurrent attacks of hemiplegia, oculogyric, and choreoathetotic involuntary movements. De novo mutations in ATP1A3 cause three types of neurological diseases: AHC; rapid-onset dystonia-Parkinsonism (RDP); and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndromes. It remains to be determined whether or not a rare mutation in ATP1A3 may cause atypical phenotypes...
2016: BMC Neurology
Natalie Smith, Jean-Marie U-King-Im, Janaka Karalliedde
We describe a case of a delayed diagnosis of Pendred syndrome. The patient had a history of hearing loss from childhood and presented to the endocrine clinic when aged 32 with an enlarging goitre. The characteristic Mondini deformity was noted on auditory canal MRI scan. Genetic tests confirmed the diagnosis. Pendred syndrome is an autosomal recessive disorder, characterised by congenital sensorineural hearing loss, goitre and impaired iodide organification and can present at any age. We highlight to the general physician the classical features of this syndrome that would aid early diagnosis...
2016: BMJ Case Reports
Hannah Glick, Anu Sharma
This review explores cross-modal cortical plasticity as a result of auditory deprivation in populations with hearing loss across the age spectrum, from development to adulthood. Cross-modal plasticity refers to the phenomenon when deprivation in one sensory modality (e.g. the auditory modality as in deafness or hearing loss) results in the recruitment of cortical resources of the deprived modality by intact sensory modalities (e.g. visual or somatosensory systems). We discuss recruitment of auditory cortical resources for visual and somatosensory processing in deafness and in lesser degrees of hearing loss...
September 6, 2016: Hearing Research
Afzal Ahmad, Benedicta D'Souza, Charu Yadav, Ashish Agarwal, Anand Kumar, M Nandini, Vivian D'Souza, A M Poornima, Nutan Kamath
Alstrom's syndrome (AS) is a rare autosomal recessive ciliopathic condition affecting 1:10,00,000 children. It's a single gene disorder of ALMS1 on chromosome 2 with multisystem involvement with cone-rod retinal dystrophy causing juvenile blindness, obesity, insulin resistance, type 2 Diabetes mellitus, hypogonadism and sensorineural hearing loss. Till now only 800 patients with this disorder has been identified so far. In this report, we describe the case of a 9-year old male boy from south India. He had been initially referred for polyphagia, polyuria, polydipsia, generalized weakness from 1 weeks...
October 2016: Indian Journal of Clinical Biochemistry: IJCB
Joel G Ray, Marian J Vermeulen, Aditya Bharatha, Walter J Montanera, Alison L Park
IMPORTANCE: Fetal safety of magnetic resonance imaging (MRI) during the first trimester of pregnancy or with gadolinium enhancement at any time of pregnancy is unknown. OBJECTIVE: To evaluate the long-term safety after exposure to MRI in the first trimester of pregnancy or to gadolinium at any time during pregnancy. DESIGN, SETTING, AND PARTICIPANTS: Universal health care databases in the province of Ontario, Canada, were used to identify all births of more than 20 weeks, from 2003-2015...
September 6, 2016: JAMA: the Journal of the American Medical Association
Luxin Yin, Zhenyu Ma, Chunde Li, Shiqi Luo
AIM: We investigated the clinical presentation and management issues of unilateral vestibular schwannomas in childhood without evidence of neurofibromatosis. MATERIAL AND METHODS: Ten pediatric patients with unilateral vestibular schwannomas and no evidence of neurofibromatosis were treated by the senior author at Tiantan Hospital from January 1994 to December 2014. The clinical manifestations, neuroimaging findings, treatment methods, and therapeutic results were retrospectively reviewed...
December 28, 2015: Turkish Neurosurgery
Carmen Barreira-Nielsen, Elizabeth Fitzpatrick, Serena Hashem, JoAnne Whittingham, Nicholas Barrowman, Mary Aglipay
OBJECTIVES: Deterioration in hearing thresholds in children is of concern due to the effect on language development. Before universal newborn hearing screening (UNHS), accurate information on the progression of hearing loss was difficult to obtain due to limited information on hearing loss onset. The objective of this population-based study was to document the proportion of children who experienced progressive loss in a cohort followed through a UNHS program in one region of Canada. We explored risk factors for progression including risk indicators, audiologic, and clinical characteristics of children...
September 2016: Ear and Hearing
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"